STIFF–PERSON VEREINIGUNG DEUTSCHLAND E.V. ⊃⊂ 52372 Kreuzau-Stockheim, Raiffeisenstraße 50 ⊃⊂ Tel. 02421 504357

Therapeutische Mglichkeiten fr Patienten mit Stiff-Person-Syndrom
Quelle: El-Abassi, Rima, et al.: SPS: Understanding the complexity. In: Journal of the Neurological Sciences 404 (2019) 137149; die [Zahlen] sind Links auf die entsprechende Fachliteratur

Class Agent Dose
Symptomatic therapy:  Benzodiazepines [33] Diazepam [28] 5-100 mg/day
Clonazepam [98, 127] 2.56 mg/day
Alprazolam 24 mg/day
Lorazepam 6 mg/day
 Muscle relaxants Tizanidine [127] 636 mg/day
Baclofen [157] 1060 mg/day
ITB [151] 50150 μg/day
Dantrolene 200400 mg/day
Botulinum toxin A [7,46] Variable
 Cannabis THC-CBD [211] Variable
 Antiepileptic  drugs Levetiracetam [179] 5001000 mg twice a day
Pregabaline 75150 mg twice a day
Gabapentin [207] 300900 mg three times day
Tiagabine [138] 48 mg once or twice a day
Valproate [190] 300600 mg twice daily
Vigabatrin [208] 5001500 mg twice daily
 Immunotherapy IVIg [56, 37] 1 g/kg body weight/month.
Rituximab [107] 2 infusions of 1 g each, every two weeks
Plasma exchange [125, 147] 5 PE in 12 weeks
Corticosteroids [68, 153] 50-60 mg/day
Mycophenolate mofetil [123] 2 g/day
Tacrolimus [142] 3 mg/day
Cyclophosphamide [209] 1-5 mg/kg/day
Azathioprine [192] 12.5 mg/kg/day
Methotrexate 15-20 mg/day
 Interventional therapy Anesthesia [182, 77]  
Spinal cord Stimulation [204]  

Rare antibodies or antibodies of unestablished significance like those against gephyrin, GABAAR, GABAAR receptor associated pr Overview of the most common immunotherapeutic strategies applied in neuronal autoantibody-associated disorders (Quelle: Balint, B. und H.M. Meinck: Pragmatic treatment of stiff person spectrum disorders. In: Mov Disord Clin Pract 5 (4), 394-401. 2018)
1st line immunotherapy
Corticosteroids Targets cellular and humoral immune responses
  • Pulse therapy with 500-1000 mg / day I.V. for 3-5 days (single / repeated)

  • Maintenance: Prednisone 1 mg/kg or 60-80 mg P.O. once daily, oral tapering

  • Co-medication for thrombosis prophylaxis (in high dose therapy), gastric ulcers (always) and osteoporosis (in chronic use)

Immediate side effects: hyperglycaemia, hypertension, leucocytosis, thrombocytosis, peptic ulceration, insomnia.
Side effects in chronic use: diabetes, osteoporosis, skin atrophy, cataracts, glaucoma
Intravenous immunoglobulins (IVIG) Blockade of cellcell interactions, neutralisation of cytokines, activated complement proteins and autoantibodies,
blockade of immune complex binding, modulation of innate immune effector cells and B cells
  • No standard dose; frequently 0.4g/kg bodyweight per day over 3-5 days as initial dose, then maintenance with 0.4g/kg bodyweight monthly; or monthly infusion of 1 g/kg bodyweight per day on 2 consecutive days

  • Co-medication for thrombosis prophylaxis

Caveat: patients with IgA deficiency (risk of anaphylactic shock); patients with nephropathy.
Immediate side effects: headache, flushing, malaise, chest tightness, fever, chills, myalgia, fatigue, dyspnoea, back pain, nausea, vomiting, diarrhoea, blood pressure changes, tachycardia;
Late side effects (rare): renal failure, thromboembolic events, aseptic meningitis, neutropenia, autoimmune haemolytic anaemia, skin reactions
Plasma exchange (PLEX) Reduction of serum levels of antibodies (and other pro-inflammatory mediators)
  • 3-6 times every other day, depending on severity
Side effects: cardiovascular stress, catheter infection, sepsis; plasma needs to be replaced by human albumin or fresh frozen plasma (risk of infections, allergic reactions).
2nd line immunotherapy
Rituximab B-cell depleting monoclonal antibody, applicable where antibodies are pathogenic
  • 1000 mg two weeks apart
  • 375 mg/m2 body surface once per week over four weeks
  • premedication:
    intravenous corticosteroids, anti-histaminics and paracetamol
Risk of severe infusion reactions;
higher risk of infections
Cyclophosphamide Targeting of cellular and humoral immune response
  • 750 mg/m2body surface once every 4 weeks I.V.

  • 500 mg per day for 24 days per month I.V.

  • monthly dose of 1000 mg I.V.

  • maximal cumulative lifetime dose 80g

Increased risk of myelo- or lymphoproliferative malignancies; urinary tract and renal toxicity (particularly haemorrhagic cystitis; therefore premedication with mesna if more than 2g I.V. or if urothelial toxicity was noticed on previous administration);
cardiotoxicity, pulmonary toxicity
Mycophenolate mofetil Inhibition of lymphocyte proliferation
  • 1000 to 1500 mg/day P.O. twice a day

  • maximal cumulative lifetime dose 140mg/m2

Gastrointestinal symptoms; myelosuppression.

Patients require periodic monitoring of blood cell count and of liver enzymes and renal function

Other drugs available for immunosuppression are e.g. azathioprine, methotrexate, cyclosporine A, mitoxantrone, and tacrolimus. Immunoadsorption is another procedure to remove antibodies and immuncomplexes from the blood; the main difference to PLEX is that the patients plasma is reintroduced in the circulation.

Important: Please note that this list is not exhaustive and does not represent treatment recommendations. Readers must always check the product information and the latest codes of conduct and safety regulations.